| | n. | % |
|---|
Classification
| | |
Primary PAP | | |
Idiopathic + autoimmune1 | 73 | 90 |
Hereditary2 | 1 | 1 |
Secondary PAP3 | 3 | 4 |
PAP-like4 | 4 | 5 |
Gender
| | |
Male | 54 | 66.5 |
Female | 27 | 33.5 |
Smoking status
| | |
Current | 18 | 22 |
Former | 34 | 42 |
Never | 29 | 36 |
- 1 We began to measure serum GMAb level in 2004, thus for accuracy in this group we included patients with idiopathic PAP (n = 42, those diagnosed before 2004) and patients with autoimmune PAP (n = 31, those diagnosed after 2004), although currently they are considered synonymous. 2 This subject was affected by PAP due to a GM-CSF receptor αchain mutation[17]. 3 This group includes two subjects with PAP secondary to hematologic disorders (1 chronic myeloid leukemia and 1 myelodysplastic syndrome) and 1 subject affected by lysinuric protein intolerance (the latter’s case history has already been reported)[18, 19]. 4 This group includes four subjects with a mutation in the ABCA3 gene (manuscript in preparation).
